Biomarkers of Development of Immunity and Allergic Diseases in Farming and Non-farming Lifestyle Infants: Design, Methods and 1 Year Outcomes in the "Zooming in to Old Order Mennonites" Birth Cohort Study.

Traditional farming lifestyle has been shown to be protective against asthma and allergic diseases. The individual factors that appear to be associated with this "farm-life effect" include consumption of unpasteurized farm milk and exposure to farm animals and stables. However, the biomarkers of the protective immunity and those associated with early development of allergic diseases in infancy remain unclear. The "Zooming in to Old Order Mennonites (ZOOM)" study was designed to assess the differences in the lifestyle and the development of the microbiome, systemic and mucosal immunity between infants born to traditional farming lifestyle at low risk for allergic diseases and those born to urban/suburban atopic families with a high risk for allergic diseases in order to identify biomarkers of development of allergic diseases in infancy. 190 mothers and their infants born to Old Order Mennonite population protected from or in Rochester families at high risk for allergic diseases were recruited before birth from the Finger Lakes Region of New York State. Questionnaires and samples are collected from mothers during pregnancy and after delivery and from infants at birth and at 1-2 weeks, 6 weeks, 6, 12, 18, and 24 months, with 3-, 4-, and 5-year follow-up ongoing. Samples collected include maternal blood, stool, saliva, nasal and skin swabs and urine during pregnancy; breast milk postnatally; infant blood, stool, saliva, nasal and skin swabs. Signs and symptoms of allergic diseases are assessed at every visit and serum specific IgE is measured at 1 and 2 years of age. Allergic diseases are diagnosed by clinical history, exam, and sensitization by skin prick test and/or serum specific IgE. By the end of the first year of life, the prevalence of food allergy and atopic dermatitis were higher in ROC infants compared to the rates observed in OOM infants as was the number of infants sensitized to foods. These studies of immune system development in a population protected from and in those at risk for allergic diseases will provide critical new knowledge about the development of the mucosal and systemic immunity and lay the groundwork for future studies of prevention of allergic diseases.

A Rare Case of Idiopathic Capillary Leak Syndrome.

Idiopathic capillary leak syndrome (ICLS) occurs as a result of vascular membrane instability, which results in the leakage of several proteins from the vascular compartment to the interstitial spaces. It is an extremely rare disorder, with around 260 cases documented thus far. We present a case of a 35-year-old male with a past medical history of asthma and gastroesophageal reflux disease who initially presented to our hospital for the treatment of chronic demyelinating inflammatory neuropathy requiring plasmapheresis and steroid therapy. After removal of his vascular catheter, he experienced sudden onset of dyspnea, hypotension, and respiratory distress. His lab work showed a hemoglobin of 21.3 g/dL and a hematocrit of 62.6%. Protein electrophoresis showed a mildly decreased albumin at 3.28 g/dL. These findings were consistent with ICLS. He required management with colloids and systemic steroids. The difficult diagnosis of ICLS is due to its overlap with several medical emergencies, such as sepsis and anaphylaxis. Further studies are required to study the role of steroids and colloids in the management of ICLS.

Lupus Cerebritis Refractory to Guideline-Directed Therapy: A Case Report.

Systemic lupus erythematosus is an autoimmune disease that affects multiple organs and organ systems, subsequently requiring an elaborate regimen for management. We present the case of a 63-year-old female who developed unrelenting symptoms of drug-induced lupus, which persisted even after the offending agent was withdrawn, unmasking her underlying systemic lupus erythematosus. She continued to develop neuropsychiatric symptoms, including mania and hallucinations, which complicated the management of her disease. After exhausting the bank of anti-inflammatory and immunomodulators recommended by current guidelines, we found that a combination of rituximab infusions with thiothixene, an antipsychotic agent, significantly improved our patient's neuropsychiatric symptoms. Further research should be conducted to determine the efficacy of rituximab in the treatment of resistant lupus cerebritis, and to validate the use of thiothixene in the management of neuropsychiatric symptoms secondary to lupus.

A Significant Pericardial Effusion Leading to Bronchial Compression: A Case Report.

Commonly, pericardial effusions can cause suboptimal heart contractility. Larger pericardial effusions can lead to compression of structures that surround in the heart in the mediastinum. Our patient presented with dyspnea that required mechanical ventilation. Bronchoscopy revealed compression of the bronchus from an external source. Echocardiogram showed a large circumferential pericardial effusion, which compressed the left main stem bronchus causing left lung atelectasis and persistent respiratory failure. A subxiphoid pericardial window was performed, which led to an improvement in her oxygen requirements. This case portrays the importance of including pericardial effusions in patients who present with respiratory failure refractory to antibiotic treatment and intervention with bronchoscopy. Although our patient passed away, recognition and earlier appropriate management with a pericardial window or pericardiocentesis could have prevented this adverse event.

An Uncommon Presentation of DRESS Syndrome Secondary to Leflunomide Use: A Case Report.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a constellation of symptoms that manifest as a result of certain medications. Several antipsychotics, antibiotics, and sulfa-containing drugs are known to be implicated in the etiology of DRESS syndrome. The clinical presentation of this disorder consists of a diffuse rash, lymphadenopathy, and systemic organ damage. Our patient presented with symptoms consistent with DRESS syndrome after being started on leflunomide, which is not commonly associated with DRESS. The diagnostic workup comprised of monitoring inflammatory markers on laboratory work, an excisional lymph node biopsy (to rule out malignancy), and a skin biopsy (to assess the etiology of the rash). Our patient received systemic steroids, dose-adjusted based on expert opinion. Further research is required to explore the association between leflunomide and DRESS and address guidelines for the management of DRESS.